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Past and current cause-specific mortality in Eisenmenger syndrome

Journal article
Authors C. M. S. Hjortshoj
A. Kempny
A. S. Jensen
K. Sorensen
E. Nagy
Mikael Dellborg
B. Johansson
V. Rudiene
G. Hong
A. R. Opotowsky
W. Budts
B. J. Mulder
L. Tomkiewicz-Pajak
M. D'Alto
K. Prokselj
G. P. Diller
K. Dimopoulos
M. E. Estensen
H. Holmstrom
M. Turanlahti
U. Thilen
M. A. Gatzoulis
L. Sondergaard
Published in European Heart Journal
Volume 38
Issue 26
Pages 2060-2067
ISSN 0195-668X
Publication year 2017
Published at Institute of Clinical Sciences
Pages 2060-2067
Language en
Links doi.org/10.1093/eurheartj/ehx201
Keywords Eisenmenger syndrome, Cause of death, Mortality, Heart failure, Pulmonary arterial hypertension, congenital heart-disease, pulmonary-hypertension, survival prospects, bosentan therapy, iron-deficiency, adults, death, guidelines, predictors, management, Cardiovascular System & Cardiology
Subject categories Cardiac and Cardiovascular Systems


Aims Eisenmenger syndrome (ES) is associated with considerable morbidity and mortality. Therapeutic strategies have changed during the 2000s in conjunction with an emphasis on specialist follow-up. The aim of this study was to determine the cause-specific mortality in ES and evaluate any relevant changes between 1977 and 2015. Methods and results This is a retrospective, descriptive multicentre study. A total of 1546 patients (mean age 38.7 +/- 15.4 years; 36% male) from 13 countries were included. Cause-specific mortality was examined before and after July 2006, 'early' and 'late', respectively. Over a median follow-up of 6.1 years (interquartile range 2.1-21.5 years) 558 deaths were recorded; cause-specific mortality was identified in 411 (74%) cases. Leading causes of death were heart failure (34%), infection (26%), sudden cardiac death (10%), thromboembolism (8%), haemorrhage (7%), and peri-procedural (7%). Heart failure deaths increased in the 'late' relative to the 'early' era (P = 0.032), whereas death from thromboembolic events and death in relation to cardiac and non-cardiac procedures decreased (P = 0.014, P = 0.014, P = 0.004, respectively). There was an increase in longevity in the 'late' vs. 'early' era (median survival 52.3 vs. 35.2 years, P < 0.001). Conclusion The study shows that despite changes in therapy, care, and follow-up of ES in tertiary care centres, all-cause mortality including cardiac remains high. Patients from the 'late' era, however, die later and from chronic rather than acute cardiac causes, primarily heart failure, whereas peri-procedural and deaths due to haemoptysis have become less common. Lifelong vigilance in tertiary centres and further research for ES are clearly needed.

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